Approach Considerations

The mainstays of medical therapy for myoclonic epilepsy are valproic acid (sodium valproate), ethosuximide, or benzodiazepines (clonazepam or clobazam).
[11] . A number of different antiepileptic medications may be efficacious, although phenobarbital, lamotrigine, vigabatrin, and carbamazepine may worsen the seizures in some cases.
[12] Combination therapy with valproic acid and benzodiazepines is often helpful. Stiripentol may have a niche role in treating Dravet syndrome.
[11, 13] In 2016, an experimental cannabis-based drug successfully treated children with Dravet syndrome. Results of the 120-patient trial showed patients taking the drug achieved a median reduction in monthly convulsive seizures of 39% compared with a reduction on placebo of 13%.
[14] More Phase III trials are forthcoming.

Adrenocorticotropic hormone (ACTH), steroids, and immunoglobulins have been tried but have not shown conclusive benefit.

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Antiepileptic Medication

Patients with benign forms of myoclonic epilepsy often respond well to valproic acid or clonazepam. Either one of the drugs may be started; if both fail independently, they may be combined. The duration of treatment is tailored on an individual basis but is usually approximately 5 years. Second-line medications include ethosuximide, zonisamide, and topiramate.

Treatment for the more severe myoclonic epilepsies is more difficult and similar to the approach used for Lennox-Gastaut syndrome. For example, patients with Dravet syndrome typically have medically refractory epilepsy and often require polytherapy.
[15]

Dietary Modification and Activity Restrictions

The ketogenic diet may be useful in children with particularly refractory epilepsy.
[16] This diet should be instituted carefully, paying particular attention to the possibility of dehydration.

Caution should be used in children with drop attacks, as they may fall and injure themselves. A helmet can be protective. Routine seizure precautions are applicable.

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Consultations and Long-Term Monitoring

Patients should be evaluated by a pediatric neurologist. If dysmorphic features are present, a genetic evaluation may be useful.

Serial EEGs often are required to ensure that patients are responding to treatment and that subclinical seizures are not occurring. Typical EEG findings in responsive patients include the disappearance of polyspike and wave activity and other associated epileptiform discharges.